Meniere's Disease
Timothy C. Hain, MD,
Page last modified:
August 17, 2006
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| Fig 1. Normal inner ear. |
What is Meniere's Disease ?
In 1861, the French physician Prosper Ménière described a condition which now bears his name. Meniere's disease is a disorder of the inner ear that causes episodes of vertigo, ringing in the ears (tinnitus), a feeling of fullness or pressure in the ear, and fluctuating hearing loss. In Figure 1, the area of the ear affected is the entire labyrinth, which includes both the semicircular canals and the cochlea.
What are the Symptoms of Meniere's Disease?
A typical attack of Meniere's disease is preceded by fullness in one ear. Hearing fluctuation or changes in tinnitus may also precede an attack. A Meniere's episode generally involves severe vertigo (spinning), imbalance, nausea and vomiting. The average attack lasts two to four hours. Following a severe attack, most people find that they are exhausted and must sleep for several hours. There is a large amount of variability in the duration of symptoms. Some people experience brief "shocks," and others have constant unsteadiness. The majority of people with Meniere's disease are over 40 years of age, with equal distribution between males and females.
A particularly disabling symptom is a sudden fall that may occur without warning. These are called otolithic crisis of Tumarkin, from the original description of Tumarkin (1936). These are attributed to sudden mechanical deformation of the otolith organs (utricle and saccule), causing a sudden activation of vestibular reflexes. Patients suddenly feel that they are tilted or falling (although they may be straight), and bring about much of the rapid repositioning themselves. This is a very disabling symptom as it occurs without warning and can result in severe injury. Often destructive treatment (for example labyrinthectomy or vestibular nerve section) is the only way to manage this problem. See here for more information about drop attacks.
Meniere's episodes may occur in clusters; that is, several attacks may occur within a short period of time. However, years may pass between episodes. Between the acute attacks, most people are free of symptoms or note mild imbalance and tinnitus.
Meniere's disease usually starts confined to one ear but it often extends to involve both ears over time so that after 30 years, 50% of patients with Meniere's have bilateral disease (Stahle et al, 1991). There is some controversy about this statistic however; some authors suggest that the prevalence of bilaterality is as low as 17% (Silverstein, 1992). We suspect that this lower statistic is due to a lower duration of follow-up and that the 50% figure is more likely to be correct. Other possibilities, however, are selection bias and different patterns of the disease in different countries. Silverstein suggested that 75% of persons destined to become bilateral do so within five years.
In most cases, a progressive hearing loss occurs in the affected ear(s). A low-frequency sensorineural pattern is commonly found initially, but as time goes on, it usually changes into either a flat loss or a peaked pattern (click here for more information about hearing testing). Although an acute attack can be incapacitating, the disease itself is not fatal.
How Common is Meniere's Disease?
Meniere's disease affects roughly 0.2% of the population. Interestingly, the Framingham study found that 2% of the population of the United States believe they have Meniere's disease, suggesting that there is considerable chance of misdiagnosis.
What Causes Meniere's Disease?
Figure 2a: Normal membranous labyrinth |
2b. Dilated membranous labyrinth in Meniere's disease (Hydrops) |
An acute attack of Meniere's disease is generally believed to result from fluctuating pressure of the fluid within the inner ear. This is called "hydrops". A system of membranes, called the membranous labyrinth, contains a fluid called endolymph. The membranes can become dilated like a balloon when pressure increases. One way for this to happen is when the drainage system, called the endolymphatic duct or sac is blocked. In some cases, the endolymphatic duct may be obstructed by scar tissue, or may be narrow from birth. In some cases there may be too much fluid secreted by the stria vascularis. Recently, some have pointed out that this "central hypothesis" of Meniere's is questionable, as many normal people without Meniere's have evidence of increased pressure in the inner ear too.
Abnormally enlarged fluid pathways into the ear, such as the vestibular aqueduct or cochlear aqueduct, may also be associated with Meniere's-like symptoms, but recent evidence is against a relationship between the cochlear aqueduct and Meniere's disease (Yilmazer et al, 2001).
Recently, attention has been mainly focussed on the immunologic function of the endolymphatic sac; thus, immune disease may contribute to a substantial percentage of Meniere's disease. However, for the most part, the underlying cause of Meniere's disease is unknown. It is most often attributed to viral infections of the inner ear, head injury, a hereditary predisposition, and allergy. Click here for more about the causes of Meniere's disease.
Meniere's disease can cause hair cell death, as well as mechanical changes to the ear.
Hair Cell Death
The periodic admixture of perilymph and endolymph often kills hair cells in the inner ear. This is a gradual process over years, but frequently resulting in unilateral functional deafness. Cochlear (hearing) hair cells are the most sensitive. Vestibular (motion sensing) hair cells seem more resilient but there is also a slow decline in the caloric response in the diseased ear over roughly 15 years (Stahle et al, 1991).
Mechanical Changes
Mechanical disruption of the inner ear is also likely, with dilation of the utricle and saccule of the ear being a well known pathological finding. The saccule may dilate so that, in later stages, it is adherent to the underside of the stapes footplate. This mechanical disruption and distortion of normal inner ear structures may result in the gradual onset of a chronic unsteadiness, even when patients are not having attacks. The periodic dilation and shrinkage of the utricle is also a reasonable explanation for periodic attacks of another inner ear disorder, BPPV. Finally, it also seems likely that there may be a rupture of the suspensory system for the membranous labyrinth. This might create some mechanical instability of the utricle and saccule and consequently some chronic unsteadiness.
There is presently no evidence that Meniere's disease kills the cochleovestibular nerve (Kitamura et al, 1997).
How is Meniere's Disease Diagnosed?
Diagnosis is based on a combination of the right set of symptoms (usually episodic dizziness and hearing disturbance); hearing tests, which document that hearing is reduced after an attack, and then gets better; and exclusion of alternative causes. The differential diagnosis is broad and includes perilymph fistula, recurrent labyrinthitis, migraine, congenital ear malformations of many kinds, syphilis, tumors, Multiple Sclerosis, etc. The process of diagnosis usually includes hearing testing (audiometry), an ENG test, several blood tests (ANA, FTA), and an MRI scan of the head. Electrocochleography (ECOG) is helpful in difficult cases.
Hearing tests often begin with showing a fluctuating low-frequency sensorineural hearing loss. Over years, this gradually progresses to a "peaked" pattern with both low- and high-tone reduction, and finally a "flat" pattern, typically 50 db loss (110 db would be completely deaf). While some people have hearing that fluctuates like this without any further symptoms of dizziness or tinnitus, in most cases, this does not progress to Meniere's disease (Schaaf et al, 2001)
How is Meniere's Disease Treated?
At the present time there is no cure for Meniere's disease, but there are ways to manage the condition and help you control symptoms. Treatment for Meniere's disease falls into the following categories:
Medications for an Acute Attack of Meniere's
Medications commonly used for an acute attack include the following:
- Meclizine (Antivert), chewable (Bonine). Dose ranges from 12.5 twice a day to 50 mg three times a day.
- Lorazepam (Ativan) 0.5 mg. Usual dose is twice a day. This medication can be taken under the tongue.
- Phenergan (orally or suppository). Usual dose is 12.5 mg every 12 hours as needed for vomiting.
- Compazine (orally or suppository). Usual dose is 5 mg every 12 hours as needed for vomiting.
- Dexamethasone (Decadron) 4 mg orally for 4 days.
Medications Used Between Attacks
Diuretics
- Dyazide (Triamterine/HCTZ)
- Klonapin 0.5 mg twice a day or as needed
- Lorazepam (Ativan) 0.5 mg twice a day or as needed
- Diazepam (Valium) 2 mg twice a day or as needed
- Meclizine (Antivert ) 12.5 mg to 25 mg as needed up to 3- to 4-times a day
Calcium Channel Blockers
- Verapamil (Calan is one brand name)
- Flunarizine/Cinnarizine (not available in the USA)
- Nimodipine (Nimotop -- generally too expensive to be practical)
Steroids (Rarely Used)
- Dexamethasone (note that intratympanic administration is controversial)
- Prednisone
Immune Suppressants (Rarely Used, see AIED)
- Methotrexate
- Steroids (see above)
- Embrel
Agents that are Controversial
- Serc (beta-histine)
- Histamine injections
- Homeopathic treatments, such as VertigoHeel
- Antiviral therapy (such as Acyclovir)
- intratympanic dexamethasone
How Do I Manage an Attack of Meniere's disease?
During an acute attack, lay down on a firm surface. Stay as motionless as possible, with your eyes open and fixed on a stationary object. Do not try to drink or sip water, as you would be very likely to vomit. Stay like this until the severe vertigo (spinning) passes, then get up SLOWLY. After the attack subsides, you will probably feel very tired and need to sleep for several hours.
If vomiting persists and you are unable to take fluids for longer than 24 hours (12 hours for children), contact your doctor. He or she can prescribe nausea medication, and/or vestibular suppressant medication. Your doctor may wish to see you. Antivert and Ativan are commonly used vestibular suppressant medications and Compazine or Phenergan are commonly used medications for nausea.
What Can be Done to Reduce the Symptoms of Meniere's disease?
Between attacks, medication may be prescribed to help regulate the fluid pressure in your inner ear, thereby reducing the severity and frequency of the Meniere's episodes. Dyazide is the most common medication for this purpose. Neptazine can be used when response to Dyazide isn't good enough. Verapamil (typical dose: 120 SR) sometimes reduces the frequency of attacks. Some physicians prescribe Histamine injections. Prednisone or other steroids (for example Decadron) are occasionally helpful in short bursts. Vestibular suppressants such as Antivert (meclizine) or Klonazepam are used on an as-needed basis.
There are a number of new medications that do not have much of a track record that can be tried under the supervision of your doctor. There also some unusual medications which are either considered "alternative" or which are available only outside the United States which might be worth considering.
The hydrops diet regimen will probably be recommended (see following). This is an important part of treatment for virtually all patients with Meniere's disease. Experience has show that STRICT adherence to this dietary regimen will result in significant improvement in most patients.
The purpose of treatment between attacks is to prevent or reduce the number of episodes, and to decrease the chances of further hearing loss. A permanent tinnitus (ringing in the ears) or a progressive hearing loss may be the consequence of long-term Meniere's disease. Hearing aids may be necessary.
Surgery and Destructive Treatments of Meniere's Disease
In extremely severe cases, treatments that deaden the inner ear such as gentamicin injections or surgery may be considered. This is a last resort for persons who have severe attacks which are disabling. At present, we favor gentamicin for most instances where destructive treatments are being considered. Injections of gentamicin are given through the ear drum, through a small hole or through a small tube. This procedure allows the doctor to treat one side alone, without affecting the other. Typically, about four injections are given over a period of one month. Dizziness may reoccur one year later, requiring another series. Although intratympanic steroids injections have also been recently used to treat Meniere's disease by Shea, the present consensus is that treatment has not yet been clearly proven to be effective or to have a reasonable scientific basis. Some studies suggest that it is no better than placebo (Silverstein et al, 1998).
Alternatively, a surgical treatment is used in which the vestibular nerve is clipped. This operation, called a vestibular neurectomy or vestibular nerve section is very effective in eliminating vertigo. While very effective, this procedure, especially the hearing-sparing variant, is presently generally felt to be of much higher risk than gentamicin injection. We presently do not recommend vestibular nerve section for Meniere's disease, except in situations where gentamicin injection has failed.
Another operation, called a labyrinthectomy, is recommended in persons who have lost all usable hearing or in whom vestibular nerve section is considered too dangerous. Again, this procedure seems most applicable to situations where gentamicin has failed.
A third operation, the endolymphatic shunt procedure, is used by some doctors to relieve pressure in the inner ear. Unfortunately, while the shunt would seem to be a logical thing to do, in most studies, the shunt procedure does not appear to be better than doing nothing (Silverstein and Rosenberg, 1992). This may be because the shunt can easily get plugged up. We do not presently recommend this procedure for our patients except in unusual situations. These may include a person who has perfectly normal hearing, or an older person who might not tolerate the gentamicin procedure described above (Pensak and Friedman, 1998; Gianoli et al, 1998). A recent trend is to make an attempt to damage the sac, in addition to improving its drainage. As the sac is the immune organ of the ear, this idea makes sense if one is attempting to immunosuppress the ear.
For bilateral Meniere's disease, when the patient is incapacitated and it cannot be determined which ear is causing the dizziness, intramuscular streptomycin (1 gm twice a day) can be given on an outpatient basis until the first sign of ototoxicity develops. This treatment can generally reduce or eliminate vertigo spells without affecting hearing. This treatment however damages the inner ear and causes bilateral vestibular paresis, which has its own set of symptoms and disability.
Surgical treatment have not been shown to preserve hearing to any greater extent than medical treatments (Kinney et al, 1996). A review of surgical treatment has recently been published by LaRouere (1996).
Hydrops Diet
The fluid-filled hearing and balance structures of the inner ear normally function independent of the body's overall fluid/blood system. In a normal inner ear, the fluid is maintained at a constant volume and contains specific concentrations of sodium, potassium, chloride and other electrolytes. This fluid bathes the sensory cells of the inner ear and allows them to function normally.
With injury or degeneration of the inner ear structures, independent control is lost, and the volume and concentration of the inner ear fluid fluctuates with changes in the body's fluid/blood. This fluctuation causes the symptoms of hydrops (pressure or fullness in the ears), tinnitus (ringing in the ears), hearing loss, dizziness and imbalance.
How Does What I Eat Affect Meniere's Disease?
Your inner ear fluid is influenced by certain substances in your blood and other body fluids. For instance, when you eat foods that are high in salt or sugar, your blood level concentration of salt or sugar increases, and this, in turn, will affect the concentration of substances in your inner ear.
People with certain balance disorders must control the amount of salt and sugar that is added to food. You must also become aware of the hidden salts and sugars that foods contain. Limiting or eliminating your use of caffeine and alcohol will also help to reduce symptoms of dizziness and ringing in the ears.
Dietary Goals
The goal of treatment is to provide stable body fluid/blood levels so that secondary fluctuations in the inner ear fluid can be avoided.
- Distribute your food and fluid intake evenly throughout the day and from day to day. Eat approximately the same amount of food at each meal and do not skip meals. If you eat snacks, have them at regular times.
- Avoid eating foods or fluids that have a high salt content. High salt intake results in fluctuations in the inner ear fluid pressure and may increase your symptoms. Aim for a diet high in fresh fruits, vegetables and whole grains, and low in canned, frozen or processed foods. A 1,000 mg sodium intake diet is usually what we recommend.
- Drink adequate amounts of fluid daily. This should include water, milk and low-sugar fruit juices (for example, cranberry or cranberry-apple). Try to anticipate fluid loss which will occur with exercise or heat, and replace these fluids before they are lost. Be cautious about the milk intake -- some individuals have food allergy and get symptoms from milk products.
- Avoid caffeine-containing fluids and foods (such as coffee, tea and chocolate). Caffeine has stimulant properties that may make your symptoms worse. Caffeine also may make tinnitus louder. Large amounts of caffeine may trigger migraine (migraine can be difficult diagnostically to separate from Meniere's disease). Chocolate is also a migraine trigger.
- Limit your alcohol intake to one glass of beer or wine each day. Alcohol may trigger migraine associated vertigo.
- Avoid foods containing MSG (monosodium glutamate). This is often present in prepackaged food products and in Chinese food. It may increase symptoms in some patients, possibly because of the link to migraine associated vertigo, and also because it contains sodium.
Where Can I Get Additional Help in Modifying My Diet?
Dietitians can help you work out a nutritional program which meets your special needs. They can also suggest ways to prepare your favorite foods for a restricted-salt or low-sugar diet. With their assistance, you'll find that modifying your eating habits can help you control the symptoms of your balance disorder.
There are several excellent books that can help in determining which foods are high in salt. Barbara Kraus's "Complete Guide to Sodium" (Signet, 1987) is a paperback book which lists most foods.
Drug Considerations
- Avoid aspirin and medications that contain aspirin. Aspirin can cause tinnitus (ringing in the ear). Nonsteroidal anti-inflammatory agents such as ibuprofen or naproxen should also be avoided when practical.
- Avoid caffeine-containing medications.Caffeine can increase tinnitus as well as have the problems mentioned above under foods.
- Pay attention to the content of all over-the-counter medications as well as drugs prescribed by other doctors.Some medications may increase your symptoms.
- Avoid cigarettes.The nicotine present in cigarettes constricts blood vessels and can decrease the blood supply to the inner ear, making your symptoms worse.
How Might Meniere's Disease Affect My Life?
Since the acute symptoms of Meniere's disease are episodic, it is important to explain to your family and friends what might happen when you have an attack. Then, if the symptoms occur when they are present, they will understand and not be overly frightened.
You may be able to protect yourself from injury if you feel that an attack is about to begin. Some attacks may occur during the night, so be sure you have a night light on; you will be relying more on vision to help maintain your balance. You will want to make sure that the path to the bathroom is free of throw rugs, furniture or other obstructions.
Many studies have documented that patients with Meniere's disease tend to have more psychological disability than the normal population, possibly including depression and/or anxiety, in reaction to their disease. It may be necessary to take antidepressants or anti-anxiety drugs, under the supervision of an appropriate health care professional.
Research Studies on Meniere's Disease
At the end of 2001, a visit to the National Library of Medicine's search engine, pubmed, revealed more than 3000 research articles concerning Meniere's disease published since 1966. In spite of this concentration of effort by the medical community, Meniere's disease remains a chronic, incurable disorder that causes progressive disability to both hearing and balance. At the American Hearing Research Foundation (AHRF), we have funded basic research on Meniere's disease in the past, and are very interested in funding additional research on Meniere's disease in the future. We are particularly interested in projects that might lead to method of stopping progression of hearing loss and the disabling attacks of dizziness. Click here if you would you would like more information about contributing to the AHRF's efforts to cure Meniere's disease.
Acknowledgments
Figures are courtesy of Northwestern University.
References and Resources
- Gianoli GJ and others. Sac-vein decompression for intractable Meniere's disease: two year treatment results. Otolarygol HNS 118:22-9, 1998
- Kinney SE, Sandridge SA, Newman CW. Long-term effects of Meniere's disease on hearing and quality of life. Am J Otol 1997 Jan;18(1):67-73.
- Kitamura K, Kaminga C, Ishida T, Silverstein H. Ultrastructural analysis of the vestibular nerve in Meniere's disease. Auris Nasus Larynx 1997;24(1):27-30.
- LaRouere MJ. Surgical treatment of Meniere's disease. Otolaryngol Clin North Am 1996 Apr;29(2):311-322.
- Pensak ML, Friedman RA. The role of endolymphatic mastoid shunt surgery in the managed care era. Am J Otol 1998 May;19(3):337-340.
- Shea JJ Jr, Ge X. Dexamethasone perfusion of the labyrinth plus intravenous dexamethasone for Meniere's disease. Otolaryngol Clin North Am 1996 Apr;29(2):353-358.
- Savastano M, Maron MB, Mangialaio M, Longhi P, Rizzardo R. Illness behavior, personality traits, anxiety and depression in patients with Meniere's disease. J Otolaryngol 1996 Oct;25(5):329-333.
- SCHAAF H, Seling B, Rienhoff NK, Laubert A, Nelting M, Hesse G. [Is recurrent loss of low frequency tone perception--without vertigo--a precursor of Meniere disease?] HNO 2001; 49: 543-7.
- Silverstein H, Rosenberg SI. Surgical techniques of the temporal bone and skull base. Philadelphia; Lea and Febinger, 1992
- Silverstein H and others. Dexamethasone inner ear perfusion for the treatment of Meniere's disease: A prospective, randomized double-blind crossover trial. Am J. Otol. 19:196-201, 1998
- Stahle J, Friberg U, Svedberg A. Long-term progression of Meniere's disease. Acta Otolaryngol (Stockh) 1991:Suppl 485:75-83
- Tumarkin A. The otolithic catastrophe: a new syndrome. Br. Med J. 1936:1:175-177
- Yilmazer C. Relationship of the cochlear aqueduct and inner ear pressure in Meniere's disease and in a normal population. Otol Neurotol 22: 534-538, 2001
Books on Meniere's Disease
Self-help Groups
- The EAR Foundation (2000 Church Street, Box 111, Nashville, TN 37236)
- Self-Help for Hard of Hearing (7800 Wisconsin Ave, Bethesda, MD 20814).
- Vestibular Disorder Association (VEDA) P.O. Box 4467, Portland Oregon 972008-4467
Other Links
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